Congenital diaphragmatic hernia.

摘要: Congenital Diaphragmatic Hernia (CDH) is defined by the presence of an orifice in diaphragm, more often left and posterolateral that permits herniation abdominal contents into thorax. The lungs are hypoplastic have abnormal vessels cause respiratory insufficiency persistent pulmonary hypertension with high mortality. About one third cases cardiovascular malformations lesser proportions skeletal, neural, genitourinary, gastrointestinal or other defects. CDH can be a component Pallister-Killian, Fryns, Ghersoni-Baruch, WAGR, Denys-Drash, Brachman-De Lange, Donnai-Barrow Wolf-Hirschhorn syndromes. Some chromosomal anomalies involve as well. incidence < 5 10,000 live-births. etiology unknown although clinical, genetic experimental evidence points to disturbances retinoid-signaling pathway during organogenesis. Antenatal diagnosis made this allows prenatal management (open correction hernia past reversible fetoscopic tracheal obstruction nowadays) may indicated severe lung hypoplasia grim prognosis. Treatment after birth requires all refinements critical care including extracorporeal membrane oxygenation prior surgical correction. best hospital series report 80% survival but it remains around 50% population-based studies. Chronic tract disease, neurodevelopmental problems, neurosensorial hearing loss gastroesophageal reflux common problems survivors. Much research on several aspects condition warranted.